Dieta Cetogénica en el paciente con epilepsia refractaria / Ketogenic Diet in patients with refractory epilepsy

INTRODUCCIÓN: La epilepsia afecta a 0,5% a 1% de la población, iniciándose en la infancia en el 60% de los casos. El 25% de los niños que la presentan, tienen epilepsia refractaria (ER) a fármacos antiepilépticos (FAE) y en ellos la dieta cetogénica (DC) surge como un tratamiento no farmacológico efectivo. OBJETIVO: Evaluar el impacto de la DC en el número de crisis, en la calidad de vida y bienestar del paciente y su entorno. PACIENTES Y MÉTODO: Se revisaron los registros médicos de los pacientes con diagnóstico de ER que recibieron DC entre los años 2008 y 2018 registrando variables: edad, diagnóstico, número de crisis, número de FAE, respuesta y complicaciones. La DC se inició en todos los casos con el paciente hospitalizado durante un período no mayor a siete días, en el cual se realizó evaluación nutricional antropométrica con medición de peso y talla según condición clínica. RESULTADOS: Se analizaron 35 DC. La mediana de edad al inicio fue 4,8 años con rango intercuartil (RIC) de 2,3-6,8 años. Se utilizó DC clásica en 49% de los pacientes, Dieta Atkins Modificada en 37% y Dieta de Bajo Índice Glicémico en 14%. Su promedio de duración fue 13 meses + 11 DS meses. Luego de tres meses de iniciada la DC, la reducción de al menos un 50% de las crisis se observó en 82% de los casos, de los cuales 22,8% presentaron reducción de más de 90% y 20% quedó libre de crisis. Se registraron efectos secundarios en 21 pacientes, la mayoría gastrointestinales (62%) y dislipidemia (14%). Todos los efectos secundarios se resolvieron con manejo médico. CONCLUSIONES: La DC es un tratamiento efectivo en pacientes pediátricos con ER y las complicaciones asociadas fueron fácilmente controla das por un equipo multidisciplinario.

INTRODUCTION: Epilepsy affects 0.5 to 1% of the population. 25% of pediatric patients have drug-resistant epilepsy (DRE). Ketogenic Diet (KD) emerges as an effective, non-pharmacological treatment in this group. OBJECTIVE: To describe the effect of KD on seizure control and nutritional status in children whit DRE. PATIENTS AND METHOD: We reviewed the medical records of patients with DRE treated with KD, between 2008 and 2018, evaluating age, diagnosis, number of seizures, number of antiepileptic drugs used, clinical outcomes, and complications. The KD was initiated in all patients hospitalized for a period no longer than seven days, who were evaluated for their nutritional and anthropometric sta tus, with weight and height measurements according to the clinical condition. RESULTS: We analyzed 35 KD in 33 cases. The median age of KD initiation was 4.8 years with an interquartile range (IQR) of 2-3 to 6.8 years. Classical KD was used in 49% of patients, Modified Atkins Diet (MAD) in 37%, and Low-Glycemic Index Treatment (LGIT) in 14% of cases. The average duration was 13 months (SD 11 months). After three months of using KD, we observed at least 50% reduction of seizures in 82% (27/33) of the patients, out of these, 22.8% presented 90% or more reduction of seizures, and 20% ended up seizure-free. Adverse events were observed in 21 patients, mainly gastrointestinal (62%) and dyslipidemia (14%), without effect on height. All side effects resolved with medical ma nagement. CONCLUSIONS: KD is a useful treatment in pediatric patients with DRE without nutritional impact. The adverse events were easily controlled if the patients are evaluated by a multidisciplinary team, according to international guidelines.

The use of a formula-based ketogenic diet in children with refractory epilepsy / O uso da dieta cetogênica a base de fórmula em crianças com epilepsia refratária

ABSTRACT The ketogenic diet (KD) is a nonpharmacologic treatment that has been used for refractory epilepsy since 1921. The KD is a high-fat, low-carbohydrate, and restricted protein diet, which is calculated and weighed for each individual patient. Introducing and maintaining the diet for a long time remains a challenge. In this study, we evaluated the acceptability, tolerance, and efficacy of a formula-based KD in 10 children with refractory epilepsy. The ketogenic formula tested herein caused only mild KD-related adverse events and adequate adherence. Moreover, 60% of patients had more than 50% seizure frequency reduction and 10% were seizure-free.

RESUMO A dieta cetogênica (DC) é um tratamento não farmacológico utilizado para epilepsia refratária desde 1921. A DC possui um alto teor de gordura, sendo restrita em carboidratos e adequada em proteínas, calculada e pesada para cada paciente. A introdução e manutenção da DC por um tempo prolongado permanece um desafio. Neste estudo foi avaliada a aceitação, tolerância e eficácia da DC baseada em fórmula em 10 crianças com epilepsia refratária. A DC apresentou adequada aderência e efeitos adversos leves. Além disso, 60% dos pacientes apresentaram mais de 50 % de redução frequência das crises e 10% ficaram livres de crises.

Ketogenic diet for epilepsy treatment / Dieta cetogênica para o tratamento da epilepsia

ABSTRACT The ketogenic diet (KD), a high-fat, low-carbohydrate, and adequate-protein diet is an established, effective nonpharmacologic treatment option for intractable childhood epilepsy. The KD was developed in 1921 and even though it has been increasingly used worldwide in the past decade, many neurologists are not familiar with this therapeutic approach. In the past few years, alternative and more flexible KD variants have been developed to make the treatment easier and more palatable while reducing side effects and making it available to larger group of refractory epilepsy patients. This review summarizes the history of the KD and the principles and efficacy of the classic ketogenic diet, medium-chain triglyceride(s) (MCT) ketogenic diet, modified Atkins diet, and low glycemic index treatment.

RESUMO A dieta cetogênica, rica em gorduras, adequada em proteínas e pobre em carboidratos é uma opção de tratamento efetiva e bem estabelecida para epilepsia de difícil controle na infância. Foi desenvolvida em 1921 e nos últimos 20 anos tem sido utilizada em diferentes países e culturas, porém ainda é desconhecida entre muitos neurologistas e outras especialidades. Recentemente, no esforço de tornar a dieta mais palatável, de fácil administração e com menos efeitos adversos, dietas cetogênicas alternativas foram criadas, possibilitando a sua administração a um maior número de pacientes. Nesta revisão abordaremos a história, príncipios e eficácia da dieta cetogênica, da dieta com triglicérides de cadeia média, da dieta modificada de Atkins e da dieta com baixos índices glicêmicos.

Neurobiochemical mechanisms of a ketogenic diet in refractory epilepsy

A ketogenic diet is an important therapy used in the control of drug-refractory seizures. Many studies have shown that children and adolescents following ketogenic diets exhibit an over 50% reduction in seizure frequency, which is considered to be clinically relevant. These benefits are based on a diet containing high fat (approximately 90% fat) for 24 months. This dietary model was proposed in the 1920s and has produced variable clinical responses. Previous studies have shown that the mechanisms underlying seizure control involve ketone bodies, which are produced by fatty acid oxidation. Although the pathways involved in the ketogenic diet are not entirely clear, the main effects of the production of ketone bodies appear to be neurotransmitter modulation and antioxidant effects on the brain. This review highlights the impacts of the ketogenic diet on the modulation of neurotransmitters, levels of biogenic monoamines and protective antioxidant mechanisms of neurons. In addition, future perspectives are proposed. .

Dieta cetogénica / Ketogenic diet

Históricamente se describió al ayuno como tratamiento de la epilepsia. La dieta cetogénica (DC), es alta enlípidos, baja en proteínas y en hidratos de carbono, es decir, se invierte el cociente normal con la finalidad deproducir y mantener un estado de cetosis. La DC debe producir cuerpos cetónicos debido a la oxidación incompleta de los lípidos. Casi todos los estudios han logrado establecer una asociación entre cetonemia yeficacia anticonvulsiva. Mientras muchos estudios han sugerido que la cetosis persistente es esencial para laprotección anticonvulsiva de la DC, otros han propuesto que la restricción de glucosa es la clave. Los PUFAs, se cree que actúan a nivel cardíaco sobre los canales de sodio y calcio, encuentros similares se han descrito en tejido neuronal.

Historically described fasting as a treatment for epilepsy. The ketogenic diet (KD), is high in fat, low inprotein and carbohydrates, that is, the normal ratio is reversed in order to produce and maintain a state ofHistorically described fasting as a treatment forepilepsy. The ketogenic diet (KD), is high in fat, low inprotein and carbohydrates, that is, the normal ratio isreversed in order to produce and maintain a state ofketosis. The KD should produce ketone bodies due tothe incomplete oxidation of lipids. Almost all studieshave established an association between ketonemia andanticonvulsant efficacy. While many studies havesuggested that persistent ketosis is essential for theprotection of the KD anticonvulsant, others haveproposed that glucose restriction is the key. ThePUFAs, are thought to act at the heart of the sodium andcalcium channels, similar events have been describedin neuronal tissue.

Ketogenic diet in indian children with uncontrolled epilepsy.

Objective: To evaluate the efficacy of the ketogenic diet in Indian children with uncontrolled epilepsy. Study Design: Prospective observational study. Setting: Hospital based. Patients: 105 children (age 4 months to 18 years) with uncontrolled epilepsy enrolled in the ketogenic diet program over a period of 9 years and followed up for 25.7 ± 20.3 months (median:17 months) on the ketogenic diet. Main outcome measures: Reduction in seizure frequency and comparison of improvement in two main groups of epilepsies, namely epileptic encephalopathies and localization related epilepsies. Results: Thirty seven (35%) out of 105 children dropped out of the study and 68 remained on the diet. Thirty nine (37%) achieved 100% control, 23 (22%) achieved between 90 and 99% control, 7 (6.8%) achieved between 75 and 90% control, and 16 (15.2%) achieved between 50 and 75% control. Twenty (19%) achieved less than 50% control. Epileptic encephalopathies had a better response than localization related epilepsies. Conclusion: The Indian version of ketogenic diet used is well tolerated and efficacious in controlling difficult-to-control epilepsy in children. Epileptic encephalopathies respond better than localization related epilepsies